Ellis-van Creveld syndrome, or chondroectodermal dysplasia, is an autosomal recessive disorder with characteristic clinical manifectations, The syndrome seems as a result of the mutattion at the 61 th band of the of the long arm of 4th chromosome. Its incidence in the general popultion is 7/1.000.000. Chondroectodermal Dysplasia is a disease complex consisting of bilateral manual polidactyly, chondrodysplasia of long bones resulting in acrornelic dwaefism, hydrotic actodermal dysplasia affecting principally the nails. teeth and hair and congential heart malformations. The presence of a great varity of oral microdontic teeth, and congenitally missing teeth requires multidisciplimanary dental treatment. with consideration for the high incidence of cardiac defects in these patients. In this article, a case of a 9 year old child with chondroectodermal dyplasia is presented. After the physical, radyologic. intra-oral and extra-oral investigations; bilateral manul polydactyly, acromelic dwarfism, distrofic nails, abnormally shaped and microdontic teeth, congenitally missing teeh is identifed. As the intra-oral treatments are done for the patient, it is considered that she needs a longer follow up.